Search

Background: Store-and-forward (SAF) tele-dermatology (TD) platforms could help promote coordination between hospital and general practitioners (GPs). However, very little data exists on the performance accuracy and opinions of GPs participating in this type of project in France., Methods: We report on the diagnostic and management plan accuracy of an SAF-TD platform developed for neighbouring GPs around our hospital compared with routine face-to-face (FTF) dermatological consultation in our department. We also compared the accuracy of SAF-TD with that of the participating GPs. Lastly, we collected feedback from GPs after their participation in this project., Results: Overall, 298 patients were included by 58 GPs between November 2016 and January 2020, of whom 169 (57%) were female, and with a median age of 44.5 years (range 0-96). The diagnostic accuracy of TD was 62% (n=184/298) for the initial hypothesis and 80% (n=239/298) for aggregated diagnostic accuracy. Management plan accuracy for TD was 81% (n=225/277). At least 43% of consultations (n=127/298) met the criteria for preventable consultation. Diagnostic accuracy for the initial hypothesis was significantly lower for GPs than for TD (Odd Ratio [OR]=0.34; 95% Confidence Interval [95% CI]: 0.20-0.56; p<0.0001), as was management plan accuracy (OR=0.23; 95% CI: 0.10-0.46; p<0.0001). Among the responding GPs, 78% (n=29) reported very high satisfaction and 97% would consider integrating this type of programme in their long-term practice, but they highlighted the time-consuming nature of the platform (46%) and the lack of financial compensation (44%)., Conclusion: SAF-TD in coordination with GPs seems safe and efficient in the management of outpatients, and enjoys a high satisfaction rate among GPs, despite its time-consuming nature and the lack of financial compensation. Healthcare policy should promote financial participation to help the expansion of TD., (Copyright © 2022. Published by Elsevier Masson SAS.)

The term Sneddon's syndrome (SS) has been used since 1965 to describe a vasculopathy characterized by a combination of cerebrovascular disease with livedo racemosa. SS may be classified as antiphospholipid+ (aPL+) or antiphospholipid- (aPL-). Little is known about aPL- SS; in this review we describe the epidemiology and pathogenesis of aPL- SS, as well as the clinical and histologic features. We discuss recent findings in terms of neurologic and cardiac involvement. Moreover, differential diagnoses of conditions that may present with both livedo racemosa and stroke are discussed. Finally, we discuss real-life practical issues such as the initial investigations to be performed, long-term follow-up, and therapeutic management of aPL- SS patients., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Cutaneous drug-induced lupus erythematosus (CDILE) is a lupus-like syndrome related to drug exposure which typically resolves after drug discontinuation. It can present as a systemic or a sole cutaneous form and different drugs may be associated with each form. CDILE pharmacoepidemiology is constantly changing. Indeed, older drugs primarily associated with systemic CDILE are no longer prescribed and new drugs associated with either cutaneous or systemic CDILE have emerged. The present study discusses the clinical and laboratory aspects of CDILE and the postulated pathogenesis, and it provides an update on implicated drugs. We performed a literature review to single out the new drugs associated with CDILE in the past decade (January 2010-June 2020). Among 109 drugs reported to induce CDILE in 472 patients, we identified anti-TNFα, proton-pump inhibitors, antineoplastic drugs, and, in particular, checkpoint inhibitors, as emerging drugs in CDILE. Most of the published studies are cases reports or small case series, and further larger studies as well as the development of validated classification criteria are needed to better understand and characterize their implication in CDILE., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

The media and social networks often echo fears about the potential toxicity of cosmetics and the dangers they pose to the environment. Dermatologists may be asked about these topics, but despite regulatory labelling constraints and the proliferation of specialized sites and applications, they do not always have access to reliable information. It is for this reason that we are providing the present overview of current knowledge on the subject., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Background: Chilblains are inflammatory dermal lesions associated with hypersensitivity to cold, and they occur on the extremities bilaterally and symmetrically. Their onset during the course of pro-thermogenic and autoimmune diseases has been widely reported, but the association with predisposing locoregional causes is not well known., Patients and Methods: Case 1: a 57-year-old man, who smoked 80 packets per year, presenting a deficit of the levator muscles in his right foot following lumbar sciatica with paralysis of L5, consulted for unilateral necrotic lesions of the toes recurring each winter in the paralysed limb only. Case 2: a 60-year-old man had a previous history of liposarcoma of the right side treated with radiotherapy and surgery, resulting in sequelae of monoparesis and radiation-induced arteritis. Each winter, he presented recurring unilateral purpuric macules of the toes on his right foot, with no necrotic progression. In both cases, clinical examination, disease progression over time, histology and laboratory tests confirmed the diagnosis of idiopathic chilblains., Conclusion: The physiopathological hypotheses posited to account for the unilateral appearance of chilblains in the event of paralysis include decreased blood flow to the paralysed limb, imbalance in neuromodulators, dysfunction of the autonomous nervous system, cutaneous atrophy with hypertrophy of underlying soft tissues, and finally, hypoesthesia aggravating the trophic disorders., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Some debate continues to surround the existence of neutrophilic urticaria (NU) as a nosological entity. Certain authors consider NU as a banal form of urticaria since an infiltrate predominantly made up of polynuclear neutrophils (PNN) is seen in certain cases of chronic and acute urticaria. Moreover, it has been stated that the histological appearance of chronic urticaria varies according to the time between appearance of the plaque and the performance of biopsy: the presence of PNN may occur later. According to the literature, there appear to be no specific clinical characteristics associated with the presence of PNN at histology. Most cases exhibit moderate laboratory inflammatory syndrome. Data concerning therapeutic response are contradictory: some studies have shown no significant difference in terms of therapeutic response in relation to banal urticaria, while only one study has demonstrated superior response to dapsone in the case of histologically demonstrated neutrophilic infiltrate. There does not appear to be any disease more frequently associated in the event of NU. In conclusion, the available data concerning NU are insufficient to confirm the existence of this condition. A prospective study comparing routine acute and chronic urticaria biopsies would be extremely useful to better characterise the relationships between cellular infiltrate and therapeutic response., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Intravascular large B-cell lymphoma (ivLBCL) is a rare blood dyscrasia that is difficult to diagnose. Healthy skin biopsies may prove useful in diagnosis of the condition. Herein we report a case of ivLBCL diagnosed using this type of examination, and we provide a literature review to determine the sensitivity of such testing., Patients and Methods: A 67-year-old woman was hospitalised for unexplained prolonged fever (UPF) and impaired general well-being. Laboratory tests revealed inflammatory syndrome, elevated LDH>2000IU/L, hepatic cytolysis and decreased prothrombin time at 47 %. Analysis for infection and medical imaging ruled out both an infectious or inflammatory origin and solid tumour. A healthy skin biopsy enabled confirmation of the diagnosis of ivLBCL., Discussion: This clinical case illustrates the value of healthy skin biopsy in establishing a diagnosis of ivLBCL in patients hospitalised for UPF. Following a systematic literature review in PubMed/Medline, we included eight studies involving at least three patients designed to assess the value of healthy skin biopsy in the diagnosis of ivLBCL. The diagnostic sensitivity of this approach ranged from 67% to 100%, with a sensitivity of 100% being seen in four of the eight studies. Details of the biopsy sites were available in three studies and diagnostic sensitivity was similar overall between samples taken from the thigh, abdomen and arms., Conclusion: Healthy skin biopsy sampling from at least two sites constitutes a sensitive and relatively non-invasive procedure for early diagnosis of ivLBCL., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Objective: Assessment of the efficacy and safety of omalizumab in chronic urticaria refractory to conventional treatment (H1-antihistamines at high dosage and montelukast) in real-life practice., Patients and Methods: A retrospective, descriptive, single-centre study was performed of the data for all patients presenting refractory chronic spontaneous urticaria or inducible urticaria and receiving omalizumab (300mg every four weeks) from November 2012 to June 2016., Results: In all, 23 patients were included. Omalizumab led to complete or significant remission in 19 patients (83%) with chronic urticaria, with remission in 9 patients (47%) occurring within 72hours of the first injection. One patient had a partial response and 3 (13%) showed no response. Only 2 patients (9%) in complete remission stopped their treatment at 1 and 3 years. 52% of patients presented non-serious adverse events, which in one case resulted in treatment withdrawal., Conclusion: Omalizumab exhibited good real-life efficacy in a small series of chronic urticaria patients in France., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: Digital necrosis is rarer than lower limb necrosis and constitutes a medical or surgical emergency. Etiological evaluation is required. Cold agglutinin disease is a cause of digital necrosis but diagnosis is difficult., Patients and Methods: Herein we report the case of a 57-year-old man presenting recent paroxysmal acrosyndrome of the left hand subsequently complicated by digital necrosis following occupational exposure to cold in his work as a forklift driver. After etiological evaluation, a diagnosis of primary cold agglutinin disease was made. Intravenous rituximab and topical treatment resulted in complete healing., Discussion: Cold agglutinin disease is a rare type of auto-immune hemolytic anemia. Following exposure to cold, paroxysmal cutaneous signs are frequent. The disease may be either primary or secondary with B-cell lymphoproliferative disorder, auto-immune disease or infection. A thorough workup is required. To date, the treatment combining the best positive response rate and good safety is rituximab in weekly perfusions over a 1-month period., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: Fingolimod is an oral immunomodulator approved for relapsing-remitting multiple sclerosis. We report a case of a primary cutaneous CD30+ T-cell lymphoproliferation occurring 6 months after initiation of fingolimod. Based on a systematic literature review, the characteristics of these fingolimod-induced lymphoproliferative disorders are described., Patients and Methods: A 56-year-old woman developed cutaneous indurated and ulcerated nodular lesions 6 months after starting fingolimod for active relapsing-remitting multiple sclerosis. Histological examination of a punch biopsy sample demonstrated a polymorphous dermal infiltrate containing large atypical CD30+ cells, leading to diagnosis of primary cutaneous CD30+ anaplastic large-cell lymphoma. Chest-abdomen-pelvis CT scans were performed to rule out secondary cutaneous anaplastic large-cell lymphoma. Spontaneous clinical regression was observed and after assessing the benefit/risk ratio, it was decided to continue fingolimod under strict surveillance, with no relapse occurring by month 18., Discussion: A systematic review of PUBMED/Medline and Embase identified seven other cases of lymphoproliferative disorders occurring during fingolimod treatment, including two other cases of primitive cutaneous CD30+ lymphoproliferative disorders., Conclusion: Even if cutaneous CD30+ lymphoproliferative disorders occur only rarely during fingolimod treatment, dermatologists should nevertheless be aware of this association for which strict dermatological surveillance is required. We would also stress that these CD30+ lymphoproliferative disorders can disappear spontaneously, as in our case, even if treatment by fingolimod is continued., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Aim: Our aim was to assess the prevalence of adverse effects (AEs) pertaining to the use and withdrawal of hydroxychloroquine (HCQ) in dermatological outpatients., Patients and Methods: We conducted a retrospective study between January 2013 and June 2014 that included consecutive patients currently or previously receiving HCQ seen in our department. AEs were collated using a standardized questionnaire and validated by clinical and laboratory examination. Drug causality was evaluated using the updated French drug reaction causality assessment method. The main evaluation criterion was the prevalence of AEs in which HCQ had an intrinsic imputability score of I>2., Results: We included 102 patients (93 of whom were women, with a median age of 44.5; range: 22-90years). HCQ was given for cutaneous lupus in most cases (n=70). At least one AE was reported for 55 patients. Among the 91 reported AEs, 59 (65%) had an HCQ intrinsic imputability score I>2. AEs were responsible for permanent HCQ discontinuation in 19 cases. Of these, 8 were unrelated to HCQ based on imputability score. The most common AEs associated with HCQ were gastrointestinal and cutaneous signs. Of the 8 patients diagnosed with retinopathy, only 3 were confirmed after reevaluation., Conclusion: AEs associated with HCQ were reported for over 50% of patients and were responsible for permanent HCQ discontinuation in one-third of cases. A more in-depth evaluation of imputability seems necessary, particularly regarding ophthalmological symptoms, since in two thirds of cases the reasons for discontinuation were not related to HCQ., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: Lipedematous scalp, with or without alopecia, is a poorly known and rarely reported entity. It was first described in 1935 by Cornbleet. It involves increased thickness of the subcutaneous tissue of the scalp, responsible for an overall thickening of the scalp, which may be associated with alopecia, pruritus or painful sensations. Currently, fewer than 50 cases of lipedematous scalp, both with and without alopecia, have been reported in the literature., Patients and Methods: Herein we present the case of a 36-year-old woman from the Ivory Coast, who presented scalp pain associated with infiltration of the entire subcutaneous tissue of the scalp seen clinically and confirmed at MRI. Histology added nothing., Discussion: We diagnosed a new case of lipedematous scalp in an African woman. No cause was found. Therapeutic abstention appears the best management strategy., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Background: The incidence of cancer is increased in patients with systemic sclerosis (SSc). Further, recent studies have also shown that the presence of anti-RNA polymerase III antibodies is associated with a higher incidence of cancer in this population., Patients and Methods: Herein we present the cases of two men aged 56 and 23 years presenting SSc without anti-Scl70 or anti-centromere antibodies but with anti-RNA polymerase III antibodies. Clinical symptoms led us to prescribe more laboratory exams and both patients were diagnosed with cancer of the nasopharyngeal area., Discussion: Anti-RNA polymerase III antibodies are useful for SSc diagnosis in patients without anti-centromere or anti-Scl70 antibodies. Their presence must lead physicians to screen for associated cancer, even in the absence of clinical signs., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Aim: The rate of hypersensitivity reactions to platinum salts (PS) and taxanes (TX) is on the increase. The aim of our study was to show the value of skin testing and efficacy of rapid drug desensitization., Patients and Methods: This was a retrospective study conducted between January 2007 and February 2016 in patients consulting for immediate or delayed hypersensitivity to PS and TX. Skin prick tests (pT) and intradermal reaction tests (IDR) were performed according to the ENDA/EAACI recommendations. We used a 12-step desensitization protocol for rapid drug desensitization., Results: Among the 99 patients included (30 men, 69 women, age 60.4) PS were suspected in 86 cases and taxanes in 13 cases. Skin tests were positive in 25 patients (7 pT, 18 IDR), 23 for platinum salts and 2 for taxanes. Rapid drug desensitization was proposed in 50 patients and performed in 33 (30 PS and 3 TX), proved effective in 29 patients, with protocol adaptation being necessary in 7 cases, and was ineffective in 4 patients. The skin tests for the latter 4 patients were positive. Seventy-five percent of patients with positive skin tests to oxaliplatin presented hypersensitivity reactions during desensitization, i.e. twice as many as patients having negative skin tests. Two percent of patient for PS and 7% for TX had cross reactivity., Conclusion: This French study confirms the efficacy of the 12-step protocol that allows patients to receive chemotherapy after hypersensitivity reaction. Skin test permits the detection of cross-reactions but their practice must be considered based on the patient's history., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Objectives: To evaluate the vaccine coverage of psoriasis patients prior to initiating or changing immunosuppressant therapy, and to verify that the prescribed vaccines have been administered., Patients and Methods: We conducted a bi-centre, observational, cross-sectional study over 9 months. Psoriasis patients in whom immunosuppressant therapy (comprising cyclosporine, methotrexate, etanercept, infliximab, adalimumab or ustekinumab) was indicated were included. Medical history, previous treatments, vaccination status, viral serology results (for hepatitis B, measles, and chickenpox), and reasons for non-vaccination were assessed via questionnaire., Results: Sixty-eight patients were included. One third brought their immunization records. Overall, 54.4% had already received immunosuppressant therapy; of these, 9 were up to date for influenza and 3 were up to date for pneumococcus. Only one patient was up to date for all of the recommended vaccinations. A total of 61% of patients were seronegative for hepatitis B. The following vaccines were updated: DTP (in 2 patients), DTP-pertussis (12), influenza (22), pneumococcus (45), and hepatitis B (6). None of the three patients with plans to travel to yellow fever-endemic countries had been vaccinated. In all, 53 (78%) stated that they had already had chickenpox and 43 (63.2%) stated that they had had one of the following three diseases: measles, rubella, or mumps. Fifty-two patients were serologically tested for chickenpox, and 98% were immunized. The most common reasons for not updating the immunization schedule were the absence of any notification or proposal by the patient's doctor and oversight., Conclusion: This study should help raise awareness among patients and health professionals concerning the new vaccination recommendations for a population particularly at risk of infection., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Background: Skin patch-tests in dermatology-allergology practice require good preparation. To this end, the dermatology-allergology group of the French Society of Dermatology introduced an information notice informing patients about patch testing procedures. The aim of this study was to evaluate the utility and understanding of the notice., Patients and Methods: The information notice was sent out to patients before testing. On the day of the test, a questionnaire was submitted to patients to evaluate their comprehension of the notice. Another questionnaire was submitted simultaneously to the dermatology-allergology practitioner to evaluate whether the patient had complied with the guidelines given in the information notice. Paired questionnaires were analyzed for this study., Results: Eight dermatology-allergology hospital departments participated in the study and collected 921 paired questionnaires over a period of 18months. Among the vast majority (96.2%) of patients who had read the information notice, most found it useful (98.8%), easy to read (97.4%), and appropriate (91.5%). Ten percent of patients had difficulty understanding., Conclusion: This study shows that the information notice was clear and explicit for the immense majority of patients. Thanks to the feedback of a number of patients, the information notice was further improved to enhance patient understanding., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Background: Dermatomyositis (DM) is an inflammatory disease associated with auto-antibodies in 50 to 70% of cases. A new antibody, anti MDA-5, has been described in association with a specific type of DM involving severe interstitial lung disease and minimal muscle disease. We report the first case of DM with MDA-5 antibodies and with interstitial lung disease and rapidly extensive digital necrosis., Patients and Methods: A 28-year-old male was hospitalized for asthenia, myalgia and subacute dyspnea. Examination demonstrated skin lesions with edema on every digit associated with purpuric and cyanotic lesions, as well as erythematous papules on the helix and the elbows, and Gottron's papules. Systemic corticosteroid therapy was initiated. The immunoprecipitation results indicated the presence of anti-MDA-5 antibodies. Despite corticosteroid therapy, the patient's respiratory status gradually deteriorated towards pulmonary fibrosis and rapidly extensive necrosis appeared on all fingers and toes. Theses effects were resistant to cyclophosphamide and immunoglobulin but were stabilized by cyclosporine., Discussion: Anti-MDA-5 antibodies are specific to DM and constitute a risk factor for severe interstitial lung disease (70% of cases) with a higher risk of mortality (40%). The cutaneous presentation of this DM is specific with palmar papules and mucocutaneous ulceration. Rapidly extensive digital necrosis has not been previously reported. No treatment has demonstrated superiority., Conclusion: We report the first case of DM with anti-MDA-5 antibodies involving interstitial lung disease and massive digital necrosis. Because of the pulmonary risk, in the presence of clinical lesions containing anti-MDA-5 DM, screening for these antibodies should be carried out., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Background: Leukocytoclastic vasculitis is characterized by necrotizing inflammation around small blood vessels, composed mainly of neutrophils. Skin lesions in leukocytoclastic vasculitis are polymorphous, but a rare annular variant exists of which dermatologists must be aware. Herein we present a new case of this entity., Patients and Methods: We report the case of a 74-year-old man who developed annular purpuric infiltrated lesions mainly on the lower limbs, with leukocytoclastic vasculitis being confirmed by histology. This annular leukocytoclastic vasculitis regressed spontaneously with no recurrence after 6 months. The aetiology was not established., Discussion: Annular leukocytoclastic vasculitis is rare, and the mechanisms underlying annularity and peripheral spread as well as the aetiology remain unexplained. This form of vasculitis with unusual clinical features may constitute a new entity in the subclass of immune complex small vessel vasculitis within the Chapel Hill classification. While the aetiology and physiopathology of this vasculitis are unknown, despite the possibility of recurrence, skin involvement appears isolated and the condition carries a good prognosis., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Background: Aquagenic palmoplantar keratoderma (APPK) is characterised by whitish oedematous papules, sometimes itchy or painful of rapid onset following immersion of the palms and/or soles in water. We report a case that was atypical in terms of the secondary appearance of persistent fine desquamation., Patients and Methods: A 6-year-old girl presented with typical APPK, present for three months and characterised by swelling, skin wrinkling, hyperhidrosis and secondary desquamation of the palms and soles, and which occurred after bathing. The patient had been treated with vinblastine, mercaptopurine and oral corticosteroids for Langerhans cell histiocytosis ongoing for six months. Physical examination revealed persistent fine desquamation of the palms and soles, occurring some time after immersion in water. There was no mutation of the CFTR gene. No similar cases could be found in the pharmacovigilance database. Topical therapy with an emollient provided some relief. Symptoms had partially resolved two months after discontinuation of chemotherapy and oral corticosteroids., Discussion: Cases of unilateral palmar damage involving unusual sites have been reported. Neither secondary desquamation nor association with Langerhans cell histiocytosis have been described. Hyperhidrosis and association with 1 or 2 CFTR mutations appear to constitute predisposing factors. There are cases of APPK related to anti-inflammatory intake. In view of the clinical course, an iatrogenic origin cannot be excluded in this case., Conclusion: Persistent fine palmoplantar desquamation may be a manifestation of APPK., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Background: Extranodal NK/T-cell lymphoma (ENKTL) is a rare form of non-Hodgkin's lymphoma and carries a poor prognosis. Depending on the primary sites of anatomical involvement, it is subcategorized into nasal or extra-nasal ENKTL. Cutaneous involvement is the second localization reported for these lymphomas., Patients and Methods: A woman was admitted for erythematous infiltrative patches on the breasts having an ulcerative course. Cutaneous histopathology showed a dense, diffuse infiltrate of atypical lymphocytes. Immunohistochemistry revealed expression of specific markers for NK-cells and of cytotoxic molecules (TIA-1, granzyme B and perforin), lack of expression of T-cell markers (except positivity of cytoplasmic CD3 and CD2), and the presence of EBV-DNA in lymphoma cells. Positron emission tomography-computed tomography revealed sub- and supra-diaphragmatic multi-organ involvement (kidneys, breasts, stomach, duodenum, lungs, pleural cavity, uterus, bones). No bone marrow infiltration was noted. PCR (polymerase chain reaction) showed high circulating levels of EBV-DNA in peripheral blood. A systemic nasal-type ENKTL was diagnosed. A chemotherapy regimen including high-dose methotrexate, oxaliplatin, gemcitabine, L-asparaginase and dexamethasone was started. Despite good initial therapeutic response, the outcome was rapidly fatal with bone marrow involvement and multi-organ failure., Discussion: Major cutaneous manifestations of ENKTL comprise erythematous infiltrative patches mimicking panniculitis or cellulitis and evolving towards ulceration or necrosis. Subcutaneous nodules may also be noted. Late diagnosis at an advanced stage accounts for the poorer prognosis in extra-nasal ENKTL. In the advanced stages, treatment is based on a chemotherapy regimen including L-asparaginase, possibly followed by autologous or allogeneic hematopoietic stem cell transplantation., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Dermatoses have been better characterized as hypochromic vitiligo in subjects with dark skin or the acquired racquet nails associated with hyperparathyroidism. The innate immunity has a central role in the new classification of auto inflammatory diseases, neutrophilic dermatoses, neutrophilic urticaria or pseudo-neutrophilic urticaria. Comorbidities have been studied e.g. cardiovascular co morbidities for psoriasis, IBD associated with hidradenitis suppurativa or neurological disorders associated with pemphigoid. Bullous pemphigoid could be renamed as cutaneous pemphigoid, it can be induced by drugs especially gliptins. Genetic predispositions are analyzed in auto inflammatory diseases, psoriasis (HLA), drug eruptions (HLA or cytokines). Telaprevir often induces rashes, which can be severe but other treatments against hepatitis C as interferon can also induce debilitating rashes, some eligible for drug tolerance induction. European guidelines for the definition, classification, diagnosis of chronic spontaneous urticaria have been published. Severe cutaneous adverse drug reactions may be associated with severe systemic symptoms such as organ involvement in AGEP or the occurrence of a shock in DRESS. Allover Europe, there is now an epidemic of contact allergies to methylisothiazolinone (MIT), contained in cosmetics, wet wipes but also in paints. MIT should be tested at 2.000 ppm in water, included in the standard series for patch tests. Its role has to be evoked in any case localized on the face or hand, airborne or generalized eczema. Among infectious diseases, skin manifestations due to Parvovirus have been specified, a febrile rash can be related to dengue infection or to Zika virus., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Background: The number of household pets increased greatly during the twentieth century, with numbers of new pets (NP, i.e. any pets other than cats and dogs) rising especially sharply over the last decade., Patients and Methods: We first of all report the case of a female patient with eczema lesions on areas skin coming into contact with a ferret, with removal of the animal resulting in wound healing, followed by two patients presenting atypical polymorphous erythema reactions induced by dermatophytes present in their pet rat., Discussion: While the most common allergies are respiratory, allergic skin reactions, both immediate and delayed, may also result from contact with these new allergens. The animal itself or its environment may be the cause., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

The number of household pets increased greatly during the twentieth century, with the numbers of new pets (NP, i.e. any pet other than cats and dogs) rising especially sharply over the last decade. Contact with such animals, whose owners do not always know how to look after them properly, expose the population to new risks such as trauma, infection and allergy. While the most common allergies are respiratory, allergic skin reactions, both immediate and delayed, may also result from contact with these new allergens. The animal itself or its environment may be the cause. Herein, we review NPs and reports of allergic dermatitis associated with them., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Background: Acquired haemophilia A (AH) is an uncommon bleeding disorder that presents as multiple, disseminated spontaneous subcutaneous bleeds. Diagnosis may be made on the basis of prolonged activated partial thromboplastin time (aPTT). The severity of the disease is associated with the low risk of haemoglobin levels and with potential links with other diseases., Observations: Two men were hospitalized for extensive and spontaneous subcutaneous hematoma. In both cases, the International Normalized Ratio (INR) was normal, but aPTT was 3 times higher than normal. Autoantibodies against coagulation factor VIII confirmed the diagnosis of AH. The patients received immunomodulatory treatment. In one patient, diffuse large B-cell lymphoma was discovered one year after successful treatment of AH., Discussion: AH may be revealed by areas of bruising, subutaneous haematomas mimicking erythema nodosum, and muscle pain. APTT results alone can prompt the biologist to screen for factor VIII inhibitors. Aside from the risk of fatal bleeding, in half of all cases, the prognosis is determined by associated disorders such as blood dyscrasias, solid tumours, autoimmune diseases, use of certain medicines and pregnancy. After treatment for bleeding complications, therapy focuses on restoring the coagulation time. The aim of immunomodulatory therapy is to stem production of autoantibodies against coagulation factor VIII., Conclusion: AH must be considered rapidly in order to reduce the risk of bleeding emergencies and to screen for potential related diseases., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Background: Hodgkin's disease has been associated with a variety of cutaneous symptoms. We report two cases of Stevens-Johnson syndrome (SJS) associated with Hodgkin's disease., Patients and Methods: Case 1: a 22-year-old man was hospitalized for a second erythematous vesicular eruption with intense mucosal involvement. Histopathological examination confirmed the diagnosis of Stevens-Johnson syndrome. He also developed enlarged cervical lymph nodes that revealed Hodgkin's disease. The latter diagnosis was followed by two recurrent rashes. Treatment consisted of systemic chemotherapy. Complete remission was obtained with no signs of cutaneous recurrence after 24 months of regular follow-up. Case 2: a 29-year-old man was admitted for a generalized erythematous and bullous rash with intense mucosal involvement. Histopathological examination confirmed the diagnosis of Stevens-Johnson syndrome. He then developed muco-cutaneous icterus that was secondary to Hodgkin's disease. Under specific hematologic treatment, no cutaneous relapse was noticed., Discussion: These cases illustrate the rare association of SSJ revealing Hodgkin's disease. In these cases, no evidence was found of infectious disease or drug-induced cutaneous effects. Only one case of toxic epidermal necrolysis associated with Hodgkin's disease had previously been reported. The link between both diseases may be immunosuppression induced by Hodgkin's disease, which could favor infection inducing SJS or secretion by tumor cells granulysin, a mediator responsible for damage to keratinocytes., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: Periodically updated, the European baseline series (EBS), first introduced in France in 1980, is an indispensable tool for the exploration of contact allergy. The aim of our study was to describe the prevalence of contact sensitization in a French centre between 1981 and 2011 to determine whether certain allergens may be deleted from the current BSE., Patients, Materials and Methods: A retrospective study was conducted in a department of dermatology-allergology to analyse the results of all EBS tests performed every 10 years, from 1981 to 2001, and annually from 2007 to 2011. Some added allergens, introduced in 2010, were also studied. Changes in allergen positivity were analysed using a Cochran-Armitage test., Results: Among the 4551 patients included for the 8-year period studied, the prevalence of positivity was 42.91%. The most common allergens in the general population were nickel sulphate (17.25%), with a constantly increasing prevalence, Myroxylon pereirae (10.68%), fragrance mix I (8.11%), cobalt chloride (6.99%) and chromium (6.33%). The least frequent sensitizations, with a decreasing prevalence, were found with clioquinol (0.25%), primin (0.54%) and benzocaine (0.55%)., Conclusion: Due to modifications in exposure to allergens, the incidence of contact sensitization can change, but nickel sulphate sensitization is increasing despite recent European directives. Allergens with less than 1% of positive results could be withdrawn from the EBS, with benzalkonium chloride, methylisothiazolinone and lavender absolute being added., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: Primary anal mucosal melanoma is rare and is associated with a poor prognosis. The observation of a case of anal melanoma at a localized stage in a woman led us to analyze recent data from the literature on therapeutic alternatives., Patients and Methods: A 49-year-old woman presented with a pigmented swelling of the anal margin that had begun three months earlier. Complete local excision of the tumour was performed with the conservation of the anal sphincters. Histological examination revealed SSM mucosal melanoma. Abdominoperineal resection was finally performed because of tumoural invasion of the lateral margins. Staging assessment was normal. Half-yearly MRI monitoring of the pelvis was proposed and at nine months no relapse was seen., Discussion: The unusual and misleading symptoms often account for the late diagnosis and poor prognosis of anal melanoma. Treatment is not well defined: local excision with conservation of the anal sphincters is recommended as first-line therapy, but the surgical technique is controversial. Abdominoperineal resection is recommended if the surgical margins are invaded, in the case of local recurrence or if the tumour is inaccessible. The place of adjuvant therapies remains to be defined. More recently, the discovery of mutation in c-KIT mucosal melanoma has allowed the use of biotherapy. Our observation underscores the importance of early detection of anal melanoma by all practitioners concerned in view of its aggressiveness and we report the difficulties of therapeutic management in the absence of established guidelines., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: Mammalian target of rapamycine (mTOR) inhibitors are being increasingly prescribed as antitumoural drugs, and associated adverse cutaneous effects are frequent but poorly described. The aim of this study was to describe such adverse effects and to assess the quality of life of patients experiencing them., Patients and Methods: Over a period of 18 months, 18 patients treated with mTOR inhibitors for renal carcinoma were included and 77 dermatological examinations performed. Wherever a cutaneous adverse event was present, quality of life was evaluated using the Skindex 30 questionnaire., Results: Fifteen of the 18 patients included presented adverse cutaneous events, consisting of buccal ulcers (61.1%), xerosis (55.5%), distal onycholysis (50%), acneiform eruption (38.8%), paronychia (22.2%) and pruritus (22.2%). Buccal ulcerations and perionyxis had an especially marked impact on quality of life, which was greatest in terms of physical score (19%), followed by emotional (9%) and functional (6%) scores., Conclusion: Cutaneous adverse effects of mTOR inhibitors are frequent and have a considerable impact on quality of life, particularly as regards physical scores. Dermatological examination appears useful to allow early management of cutaneous adverse effects and improve the quality of life of these patients., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: Mucosal erosions in bullous diseases and leading to mucosal sequelae are widely described in toxic epidermal necrolysis (TEN). These complications cause disfigurement and functional impairment. They are more rarely reported in erythema multiforme (EM). We report a case of lip adhesion following EM induced by Mycoplasma pneumoniae., Patient and Methods: A 12-year-old boy was hospitalized in a paediatric intensive care unit. He had a prominent target skin rash on the palms and soles. Mucosal injury was associated with conjunctivitis, balanitis without dysuria and hyperalgesic stomatitis. M. pneumoniae serology was positive with immunoglobulin M. We made a diagnosis of EM secondary to M. pneumoniae infection. Two months later, the skin lesions had completely disappeared but the patient's mouth opening was limited to 25 mm and he presented bilateral adhesions between the upper and lower lips of 5mm on the right and 8mm on the left resulting in aesthetic and functional damage., Discussion: Mucosal damage and its sequelae have been widely described in TEN. Ophthalmic sequelae are more frequent. A case of labial synechiae secondary to TEN has been reported. In EM, mucosal lesions occur in 100% of cases with a further mucosal problem being present in 50% of patients. Mucosal damage has been reported during EM flares but there are no studies of side-effects after the acute episode. Oral mucosal adhesions can cause cosmetic sequelae, but above all they hinder functional prognosis. These complications must be prevented by making gutters of vestibular deepening and lip movements with maximum mouth opening several times a day, starting as soon as possible. Appropriate pain management should be undertaken to ensure patient comfort and avoid the need for analgesics and restriction of movement., Conclusion: Mucosal sequelae exist in EM. Whatever their cause, complications involving the mucosa must be prevented through early, tailored and multidisciplinary treatment. Adequate pain management must not be overlooked., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Introduction: In 2007, Petrella et al. identified a new entity: CD8 T-cell indolent lymphoma of the ear., Case Report: A 40-year-old man presented a nodular erythematous and violaceous painless lesion on his right ear that had appeared four months earlier. Excision histology revealed a non-epidermotropic T-cell proliferation infiltrating the entire dermis and subcutis but with sparing of a grenz zone. The monotonous infiltrate was positive for CD8, CD3, CD5 and TIA-1, and negative for CD30, CD4, CD56, ALK and EMA. The Mib1 proliferation index was 20%. Lyme serology and PCR for EBV were negative. Additional examinations showed no extracutaneous involvement., Discussion: CD8+ indolent lymphoma is an entity first described in 2007 and reported in the literature in 15 patients. Lesions are located on the nose or external ear. It comprises a non-epidermotropic proliferation of CD8+ T lymphocytes negative for CD4, CD30, CD56, CD57, granzyme B and perforin. The Mib1 proliferation index is low. This new entity appears neither in the 2005 World Health Organization/European Organization for Research and Treatment of Cancer (WHO/EORTC) classification of cutaneous lymphomas nor in the WHO 2008 Classification of tumours of haematopoietic and lymphoid tissues. Surgical treatment or radiotherapy is sufficient, and unlike aggressive, epidermotropic CD8+ T lymphomas chemotherapy is not required., (Copyright © 2012. Published by Elsevier Masson SAS.)

Background: Multiple-drug hypersensitivity (MDH) in the literature concerns different entities. Our objective was to define its frequency and characteristics in patients examined for cutaneous adverse drug reaction (CADR) before studying genetic predisposition., Materials and Methods: From a database comprising all patients referred for CADR between 2000 and 2010, we selected those meeting the following criteria: sensitisation to at least two chemically unrelated substances, as confirmed by positive skin tests or challenge tests. The following were excluded: patients with haematological diseases, HIV or chronic wounds and sensitization to the excipients., Results: Of the 1925 patients included, 11 (0.6%) were classed as polysensitized: eight women and three men, of mean age 62 years, presenting 2.5 episodes of drug hypersensitivity per patient. Four cases of DRESS were noted., Discussion: The strict criteria stipulated for this study enabled us to select patients with MDH, and to affirm that while it does in fact exist, it seems rare. Compared to polysensitized patients described in the literature, we preferred to distinguish between three groups of MDH: one occurring with different substances in separate episodes of CADR, one occurring with different substances during the same episode of CADR, and one occurring during DRESS and correlating with viral replication., Conclusion: MDH exists and genetic predisposition could be investigated by studying cytokine polymorphism in such patients. However, because of its rarity, it is impossible to rule out fortuitous association of two episodes of CADR in the same patient., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Background: Percutalgine(®) (dexamethasone acetate, salicylamide and hydroxyethyl salicylate) is a topical drug marketed for treatment of benign joint conditions such as mild tendinitis, small joint arthritis and sprains. The aim of the study was to describe the cutaneous side effects of Percutalgine(®) in terms of clinical signs, seriousness and causal relationship of the different components., Methods: We extracted from the French Pharmacovigilance database all cases of adverse skin reactions occurring after application of Percutalgine(®) and reported for the period between 1st January 2000 and 31st October 2010. The only files selected were those in which Percutalgine(®) was the sole suspected drug and/or allergological tests were positive for Percutalgine(®) or its components., Results: Fifty-three cases were ultimately retained and analysed. The main cutaneous side effect of Percutalgine(®) (n=41) was contact dermatitis with secondary extension in 15 cases. Onset was immediate in 12 cases, delayed in 32 cases and unspecified in eight cases. Twelve patients were hospitalized for inefficiency of the symptomatic treatment, extended lesions or generalized associated signs. Allergological tests were described in 14 cases and were positive for Percutalgine(®) (eight cases), hydroxyethyl salicylate (seven cases), salicylamide (six cases), dexamethasone (three cases), and propylene glycol (two cases)., Conclusion: Cutaneous side effects with Percutalgine(®) appear to be rare or infrequently reported. They consist chiefly of contact allergy. The component responsible for the reaction can be determined using allergological patch tests., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Background: An association between herpes virus reactivations and Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is accepted. We report six cases of DRESS with viral reactivation occurring within a single 1-month period. We attempted to find a common factor for these six cases and carried out clinical and virological examinations. Before and after this "epidemic", the mean number of cases of DRESS seen at the same centre was one per quarter, making the occurrence of six cases within a single month all the more remarkable and prompting us to seek an explanation., Patients and Methods: All six patients had taken a partly causative medication from different drug classes three to six weeks prior to the start of symptoms and herpes virus was detected in the blood of all of these subjects at the time of DRESS onset (four reactivations and two primary infections), and one patient subsequently displayed herpetic meningoencephalitis 95 days after the initial episode, associated with recurrence of DRESS., Discussion: There was no common denominator among these six DRESS patients in terms of either drug class or reactivation of a particular type of herpes virus, which raises the possibility of a single unidentified environmental agent. DRESS does not appear fully explainable in terms of a cellular response to drug antigens but seems rather to result from complex interactions between the drug-induced immune response, viral reactivation and antiviral immune response. Several investigators have reported sequential reactivation of herpes viruses in DRESS. A viral epidemic could thus cause a "DRESS epidemic" in patients on medication., Conclusion: These cases point to the possible existence of a shared initial environmental factor (infectious or not) that favours reactivation of herpes viruses and induces DRESS in patients on medication. Before and after this "DRESS epidemic", about one patient each quarter was admitted to hospital for DRESS., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Background: Pityriasis rubra pilaris (PRP) following vaccination is rarely described in the literature. We report a case of PRP occurring two weeks after measles-mumps-rubella (MMR) vaccination., Case Report: A 17-month-old infant was referred for a rash appearing two weeks previously. The child was presenting diffuse erythematous scaly exanthema with follicular papules and orange palmoplantar keratoderma. The clinical features were highly evocative of PRP. The histology was non-specific, displaying epidermal acanthosis with a regular and thick parakeratosis, and without any impairment of the follicular infundibulum. An MMR vaccination had been given two weeks before onset of the rash. Treatment with topical corticosteroids and emollients proved effective., Discussion: Post-vaccinal PRP is rarely described in the literature. We report only the 3rd case. The first case concerned a 32-year-old woman presenting two episodes of PRP 10 days after diphtheria-tetanus-polio vaccination. The second case concerned a 47-year-old woman presenting PRP 18 days after anti-influenza vaccination and requiring treatment with acitretin. No cases have been described with MMR. These three vaccines (DTP, Tetragrip and ROR) have no shared pharmacological constituents, and the trigger mechanism could be immunological or parainfectious., Conclusion: Questioning about recent vaccination during history taking appears necessary to assess the importance of this trigger factor as well as the mechanism responsible for the onset of PRP., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Background: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis with multiple organ involvement affecting middle-aged adults. A case of ECD associated with Langerhans cell histiocytosis (LCH) is reported herein., Case Report: A 75-year-old woman presented maculopapular skin lesions on her trunk, associated with constrictive pericarditis and pleurisy present for 1 year. The skin biopsy militated in favour of LCH since it revealed a histiocytic infiltrate with a positive CD1a marker at immunohistochemistry (IHC). The association with ECD was diagnosed on the basis of pericarditis, periaortitis, pleurisy, pulmonary involvement and retroperitoneal fibrosis. The patient was treated with interferon-α2a with good initial results, but died from septic shock a year and a half later, a few months after discontinuing interferon due to poor tolerability., Discussion: The clinical, radiographic and histological arguments in favour of ECD clearly differ from those for LCH. However, as already reported, the two illnesses may be associated, thus underlining the possible existence of a link between these two histiocytic proliferations emanating from the same medullary precursor. Two hypotheses have been advanced in an attempt to explain this association: the first involves a stimulus that might lead to independent proliferation of the two cell lines while the second suggests the existence of a transformation pathway from one form of proliferation to the other., Conclusion: Screening for associated ECD should be routinely performed in patients presenting LCH with signs evocative of ECD., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)